Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): A 10-year experience Journal Article
| Authors: | Vauthey, J. N.; Woodruff, J. M.; Brennan, M. F. |
| Article Title: | Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): A 10-year experience |
| Abstract: | Background: Prior studies have shown a high rate of local recurrence and a dismal overall prognosis in malignant peripheral nerve sheath tumors (MPNSTs). Methods: Thirty-three patients who had undergone primary treatment for localized extremity MPNSTs between 1982 and 1992 were reviewed. These cases were derived from a prospective database of 890 adult extremity soft-tissue sarcomas (STS). MPNSTs were compared with other extremity STS. Results: MPNSTs were more often high grade and deep compared with other extremity STS (94% vs. 72% [p=0.009] and 97% vs. 76% [p=0.01], respectively). Location (upper or lower extremity), size (>5 cm vs. ≤5 cm), and status of margins after surgical resection (positive or negative for disease) did not differ between the two groups. When deep and high-grade MPNSTs were compared with deep and high-grade STS, a more aggressive local treatment was applied to MPNSTs with a higher number of amputations for MPNSTs (32%) compared with STS (9%; p<0.001). In order to obtain adequate margins, 16 of 21 MPNSTs arising from major nerves required either amputation (n=8) or nerve resection (n=8). Adjuvant radiotherapy was used in 48% of deep and high-grade MPNSTs, and 3-year local disease-free survival was 70%. Survival of deep and high-grade MPNSTs was comparable with other deep and high-grade STS (3-year survival 50% vs. 69%, respectively; p=0.1). Conclusion: MPNSTs show adverse clinicopathologic features compared with other STS. However, when treated aggressively, MPNSTs have an outcome similar to other deep and high-grade extremity STS. © 1995 The Society of Surgical Oncology, Inc. |
| Keywords: | survival; adolescent; adult; treatment outcome; aged; disease-free survival; adjuvant therapy; comparative study; radiotherapy, adjuvant; follow up; follow-up studies; prospective study; prospective studies; neurofibromatosis; pathology; sarcoma; amputation; surgery; nerve sheath tumor; soft tissue neoplasms; soft tissue tumor; leg; middle age; arm; nerve sheath tumors; human; male; female; article; support, u.s. gov't, p.h.s.; neurofibrosarcoma |
| Journal Title: | Annals of Surgical Oncology |
| Volume: | 2 |
| Issue: | 2 |
| ISSN: | 1068-9265 |
| Publisher: | Springer |
| Date Published: | 1995-03-01 |
| Start Page: | 126 |
| End Page: | 131 |
| Language: | English |
| DOI: | 10.1007/bf02303627 |
| PUBMED: | 7728565 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Article -- Export Date: 28 August 2018 -- Source: Scopus |
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